Updated: 11/6/2018

Soft Tissue Sarcoma

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Introduction
  • Soft tissue sarcomas are rare
    • 7000 new case/year in the United States
  • Proper evaluation is critical
    • unplanned excision of a soft-tissue sarcoma may negatively impact patient outcome   
    • delay in diagnosis can adversely affect the ultimate outcome
    • may be confused with more common pathology 
  • Metastasis
    • the lung is the most common site of metastasis
  • Prognosis
    • poor prognostic factors include
      • high grade
      • size > 5 cm
      • tumor location below the deep fascia
Classification
  • Over 50 histological types of soft tissue sarcomas exist
    • previously tested soft tissue sarcomas include
      •  synovial sarcoma
      •  liposarcoma
      •  rhabdomyosarcoma
      •  fibrosarcoma
      •  leiomyosarcoma
      •  angiosarcoma
      • alveolar soft part sarcoma (ASPS)
Presentation
  • Symptoms
    • enlarging painless mass 
  • Physical exam
    • palpable soft tissue mass
      • differentiation of whether a mass is above or below fascia has prognostic importance 
      • size greater than 5cm in cross-section is a poor prognostic factor
Imaging
  • Radiographs
    • obtain plain radiographs in two planes
  • MRI
    • is mandatory to evaluate soft tissue lesions in the extremities and determine treatment algorithm
    • diagnostic MRI
      • MRI is typically diagnostic for the following benign lesions
        • lipoma topic 
        • neurilemoma (schwannoma) topic 
        • intramuscular myxoma topic
      • if MRI is diagnostic and the mass is benign and symptomatic, then it can be removed without a biopsy
    • indeterminate MRI
      • if MRI is indeterminate or suggestive of sarcoma, then a core needle or open biopsy must be obtained before further treatment is initiated 
      • soft tissue sarcomas can look similar to hematomas so be cautious of a "hematoma" which occurs without trauma
Evaluation
  • Histology   
    • determined by type of sarcoma
      • synovial sarcoma  
      • liposarcoma 
      • rhabdomyosarcoma 
      • fibrosarcoma 
Treatment
  • Operative
    • radiation therapy & wide surgical resection   
      • indications
        • the standard of care in most cases
        • treatment must be based on a tissue diagnosis unless images are diagnostic (e.g. lipoma) 
      • radiation therapy
        • an important adjunct to surgery decreasing local recurrence
        • 50-60 Gy is the standard dose for soft-tissue sarcomas 
        • radiotherapy may be given pre- or postoperatively
          • pre-operative radiotherapy is associated with a 30% risk for wound complications
          • post-operative radiotherapy is associated with greater radiation-induced morbidity and an increased risk of radiation-induced sarcoma
      • chemotherapy
        • controversial for soft tissue sarcomas
      • surgical resection
        • must confirm border free of disease with histology
    • surgical resection of lung metastases (wedge resection or lobectomy)
      • indications
        • first line for pulmonary metastases if the preop evaluation shows that complete resection is possible
      • results
        • long-term survival is possible for select patients
Complications
  • Recurrence
    • following resection the most common location for recurrence of a low grade, soft tissue sarcoma is locally 
  • Unplanned excision 
    • CT chest, abdomen, pelvis to exclude metastasis
    • MRI of the limb to determine degree of contamination, post-operative changes, and to assess margins
    • revision surgery
      • limb salvage + radiation therapy
      • amputation
 

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Technique Guides (1)
Questions (25)
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(OBQ09.69) A 24-year-old male presents to your office with ecchymosis and a mass in the posterolateral aspect of his thigh. He states he noticed the mass approximately 6-months ago and that it has slowly been growing. He denies constitutional symptoms, is otherwise healthy, and denies any associated trauma. MRI report suggests the mass may be an organizing hematoma. Core needle biopsy in the office yields only blood. What is the next appropriate treatment option? Review Topic

QID: 2882
1

Conservative treatment - rest, ice, compression, elevation

8%

(111/1326)

2

Repeat core needle biopsy

5%

(67/1326)

3

Consult interventional radiography to place a drain under image guidance

3%

(41/1326)

4

Irrigation and debridement with initiation of postoperative antibiotics

2%

(28/1326)

5

Open surgical biopsy

81%

(1075/1326)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(OBQ04.106) A 52-year-old male presents with 6 months of swelling and pain in his leg. He states the lesion has not changed in size for several months and doesn't bother him. He is otherwise healthy and has no other complaints. Representative photograph and MRI are shown in Figures A through C. What is the best initial step in his management? Review Topic

QID: 1211
FIGURES:
1

Follow-up in 6 months with repeat radiographs

1%

(7/788)

2

Biopsy

91%

(717/788)

3

Marginal excision

3%

(25/788)

4

Radiation therapy

0%

(2/788)

5

Neoadjuvant chemotherapy and wide excision

4%

(32/788)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(OBQ05.231) Sixty Gy is a typical radiation dose for which of the following indications? Review Topic

QID: 1117
1

Prevention of post-operative heterotopic ossification

30%

(153/516)

2

Neoadjuvant radiation therapy for a high-grade chondrosarcoma

6%

(31/516)

3

Adjuvant radiation therapy for a high-grade osteosarcoma

10%

(52/516)

4

Adjuvant radiation therapy for a soft-tissue sarcoma

42%

(219/516)

5

Palliative therapy for a painful adenocarcinoma metastasis in the thoracic spine

10%

(54/516)

ML 4

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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(OBQ04.184) What is the correct treatment for an incompletely excised high grade superficial sarcoma on the volar aspect of the forearm? Review Topic

QID: 1289
1

Chemotherapy

1%

(8/1407)

2

Radiation therapy

4%

(61/1407)

3

Marginal resection of the tumor bed to spare the neurovascular bundles

4%

(59/1407)

4

Wide resection of the tumor bed and coverage as necessary

90%

(1269/1407)

5

Palliative care

0%

(3/1407)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(OBQ12.57) A 58-year-old man presents to your clinic with a 4-cm deep mass in his left thigh. It has been slowly expanding over the last 6 months. Radiographs show no evidence of bony involvement or soft tissue calcifications within the lesion. Which of the following is most appropriate at this time? Review Topic

QID: 4417
1

Repeat clinical examination and radiographs in 6 months

1%

(45/3504)

2

Contrast-enhanced MRI scan

88%

(3100/3504)

3

Fine needle aspiration

2%

(77/3504)

4

Core biopsy

4%

(155/3504)

5

Excisional biopsy

3%

(110/3504)

ML 1

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PREFERRED RESPONSE 2

(OBQ06.248) Which treatment factor has been shown to be most important for five-year disease-free survival in patients with subcutaneous sarcomas? Review Topic

QID: 259
1

Dose of chemotherapy

1%

(5/363)

2

Addition of adjuvant radiation

19%

(69/363)

3

Size of operative margin

77%

(279/363)

4

Length of chemotherapy course

1%

(5/363)

5

Addition of radiofrequency ablation

1%

(3/363)

ML 2

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PREFERRED RESPONSE 3

(OBQ06.225) A surgeon resects a small, superficial, mobile lesion from the thigh. Pathological review of the specimen suggests it is a low-grade soft tissue sarcoma with positive surgical margins. After staging the patient, no other sites of disease are identified. What is the next most appropriate treatment for this patient? Review Topic

QID: 236
1

Observation

3%

(36/1271)

2

Systemic chemotherapy

3%

(36/1271)

3

Radiotherapy

7%

(91/1271)

4

Tumor bed re-excision

86%

(1099/1271)

5

Amputation

0%

(6/1271)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(OBQ11.234) A 56-year-old male undergoes resection of a mass that was suspected to be a simple lipoma. The mass was contained within his sartorius muscle and shown in Figure A. The final pathology came back as a high grade soft tissue sarcoma. The treating surgeon operated completely within the sartorius compartment and did not violate any other compartments, however the final pathology showed positive margins. Staging studies show isolated disease in his right thigh. What is the most appropriate treatment for this patient? Review Topic

QID: 3657
FIGURES:
1

Amputation

1%

(37/2723)

2

Systemic chemotherapy

1%

(30/2723)

3

Radiation therapy

1%

(27/2723)

4

Wide excision of the tumor bed

23%

(636/2723)

5

Wide excision of the tumor bed followed by radiation therapy

73%

(1985/2723)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(OBQ04.260) A 39-year-old male is diagnosed with myxoid liposarcoma of the thigh. Radiation therapy is being considered in adjunct to surgical excision. Which of the following is true regarding radiation therapy in soft tissue sarcoma? Review Topic

QID: 1365
1

Compared to post-operative radiation, pre-operative radiation is associated with a reduced rate of wound dehiscence

7%

(92/1290)

2

Radiation therapy reduces the risk of local recurrence

74%

(950/1290)

3

Radiation therapy improves overall survival

8%

(107/1290)

4

Compared to post-operative radiation, pre-operative radiation is associated with increased rates of radiation induced morbidity such as post-radiation fibrosis

10%

(126/1290)

5

Radiation therapy decreases the chances of successful limb-salvage

1%

(8/1290)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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